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Table of Contents Approach to Nonmalignant [eukopenia Thalassemia 24 and Leukocytosis Anemiaof Kidney r,;; ........ 25 Lymphopenia and Neutropenia. . . . 1 Cobalamin Deficiency. 26 Neutrophilia, Lymphocytosis, and Monocytosis 2 Folate Deficiency. . . 26 Hemoll.tic Anemias. 27 Hematopoietic Stem Cells and Their Disorders Intrinsic Erythrocyte Abnormalities 27 Overview ...........2 Extrinsic Erythrocyte Abnormalities 32 BoneMarrowFailureSyndromes .....3 AplasticAnemia ........3 lron Overload Syndromes PureRedCellAplasia ...........3 Primary/Hereditary Hemochromatosis 34 MyeloproliferativeNeoplasms .... . ... . . . . . . . 3 Secondary Iron Overload 35

BoneMarrowFailureSyndromes .....3 AplasticAnemia ........3 lron Overload Syndromes PureRedCellAplasia ...........3 Primary/Hereditary Hemochromatosis 34 MyeloproliferativeNeoplasms .... . ... . . . . . . . 3 Secondary Iron Overload 35 ChronicMyeloidLeukemia ......4 PolycythemiaVera... ...........5 Platelet Disorders EssentialThrombocythemia .. .......... 5 Normal Platelet Physiologr 35 PrimaryMyelofibrosis ...........7 Approach to the Patient With Thromboc)'topenia 36 Eosinophilia and Hypereosinophilic Syndrome . . . 7 Thrombocytopenic Disorders . . . . 36 Mastocy.tosis. ........... B Decreased Production. 36 MyelodysplasticSyndromes...... ...........8 lncreased Destruction. 36 Acute Leukemias.... ........9 Qualitative Platelet Disorders 40 AcuteMyeloidLeukemia ........9 Acquired Platelet Dysfunction . 40 AcuteLymphoblasticLeukemia. ........ 10 Platelet Function Testing 4t HematopoieticGrowthFactors ...... 11 Hematopoietic Stem Cell Transplantation Bleeding Disorders andCellularTherapies ......11 Normal Hemostasis. 47 Evaluation of Patients With Suspected Multiple Myeloma and Related Disorders Bleeding Disorders 4I Overview 12 Congenital Bleeding Disorders 42 Evaluation for Monoclonal Gammopathies . 12 Hemophilia A and B and Other Factor Monoclonal Gammopathy of Undetermined Deficiencies 42 Significance 13 Von Willebrand Disease 43 Multiple Myeloma 15 Acquired Bleeding Disorders 44 Clinical Manifestations and Findings . . 15 Coagulopathy of Liver Disease. 44 Diagnosis and Prognosis. . . . . . 15 Disseminated Intravascular Coagulation 44 Treatment. 76 Vitamin K Deficiency 45 Immunoglobulin Light-Chain Amyloidosis . T7 AcquiredvonWillebrand Disease. . . . . . 45 Waldenstr0m Macroglobulinemia . 19 Acquired Hemophilia 45 Cryoglobulinemia . . 79

ChronicMyeloidLeukemia ......4 PolycythemiaVera... ...........5 Platelet Disorders EssentialThrombocythemia .. .......... 5 Normal Platelet Physiologr 35 PrimaryMyelofibrosis ...........7 Approach to the Patient With Thromboc)'topenia 36 Eosinophilia and Hypereosinophilic Syndrome . . . 7 Thrombocytopenic Disorders . . . . 36 Mastocy.tosis. ........... B Decreased Production. 36 MyelodysplasticSyndromes...... ...........8 lncreased Destruction. 36 Acute Leukemias.... ........9 Qualitative Platelet Disorders 40 AcuteMyeloidLeukemia ........9 Acquired Platelet Dysfunction . 40 AcuteLymphoblasticLeukemia. ........ 10 Platelet Function Testing 4t HematopoieticGrowthFactors ...... 11 Hematopoietic Stem Cell Transplantation Bleeding Disorders andCellularTherapies ......11 Normal Hemostasis. 47 Evaluation of Patients With Suspected Multiple Myeloma and Related Disorders Bleeding Disorders 4I Overview 12 Congenital Bleeding Disorders 42 Evaluation for Monoclonal Gammopathies . 12 Hemophilia A and B and Other Factor Monoclonal Gammopathy of Undetermined Deficiencies 42 Significance 13 Von Willebrand Disease 43 Multiple Myeloma 15 Acquired Bleeding Disorders 44 Clinical Manifestations and Findings . . 15 Coagulopathy of Liver Disease. 44 Diagnosis and Prognosis. . . . . . 15 Disseminated Intravascular Coagulation 44 Treatment. 76 Vitamin K Deficiency 45 Immunoglobulin Light-Chain Amyloidosis . T7 AcquiredvonWillebrand Disease. . . . . . 45 Waldenstr0m Macroglobulinemia . 19 Acquired Hemophilia 45 Cryoglobulinemia . . 79 Iransfusion Medicine Erythrocyte Disorders Blood Donor Screening 45

ChronicMyeloidLeukemia ......4 PolycythemiaVera... ...........5 Platelet Disorders EssentialThrombocythemia .. .......... 5 Normal Platelet Physiologr 35 PrimaryMyelofibrosis ...........7 Approach to the Patient With Thromboc)'topenia 36 Eosinophilia and Hypereosinophilic Syndrome . . . 7 Thrombocytopenic Disorders . . . . 36 Mastocy.tosis. ........... B Decreased Production. 36 MyelodysplasticSyndromes...... ...........8 lncreased Destruction. 36 Acute Leukemias.... ........9 Qualitative Platelet Disorders 40 AcuteMyeloidLeukemia ........9 Acquired Platelet Dysfunction . 40 AcuteLymphoblasticLeukemia. ........ 10 Platelet Function Testing 4t HematopoieticGrowthFactors ...... 11 Hematopoietic Stem Cell Transplantation Bleeding Disorders andCellularTherapies ......11 Normal Hemostasis. 47 Evaluation of Patients With Suspected Multiple Myeloma and Related Disorders Bleeding Disorders 4I Overview 12 Congenital Bleeding Disorders 42 Evaluation for Monoclonal Gammopathies . 12 Hemophilia A and B and Other Factor Monoclonal Gammopathy of Undetermined Deficiencies 42 Significance 13 Von Willebrand Disease 43 Multiple Myeloma 15 Acquired Bleeding Disorders 44 Clinical Manifestations and Findings . . 15 Coagulopathy of Liver Disease. 44 Diagnosis and Prognosis. . . . . . 15 Disseminated Intravascular Coagulation 44 Treatment. 76 Vitamin K Deficiency 45 Immunoglobulin Light-Chain Amyloidosis . T7 AcquiredvonWillebrand Disease. . . . . . 45 Waldenstr0m Macroglobulinemia . 19 Acquired Hemophilia 45 Cryoglobulinemia . . 79 Iransfusion Medicine Erythrocyte Disorders Blood Donor Screening 45 Approach to Anemia. 19 Blood Group Antigens, Pretransfusion Compatibility Testing, and the Direct Antiglobulin Test 46 Anemia Due to Erythrocyte Underproduction or Maturation Defects 2t Blood Components . 46 IronDeficiency.... 21 Plasma Derivatives and Recombinant Coagulation Factors. 47 Anemia of Inflammation . . . 23

Approach to Anemia. 19 Blood Group Antigens, Pretransfusion Compatibility Testing, and the Direct Antiglobulin Test 46 Anemia Due to Erythrocyte Underproduction or Maturation Defects 2t Blood Components . 46 IronDeficiency.... 21 Plasma Derivatives and Recombinant Coagulation Factors. 47 Anemia of Inflammation . . . 23 tx

BloodManagement.. .......47 Anticoagulants. 59 TransfusionComplications..... ....47 Parenteral. 59 HemollticReactions. ..........47 Oral ...... 60 NonhemolyticTransfusion Reactions. . . . . . . . . . . 49 InfectiousComplications..... .........49 Hematologic lssues in Pregnancy Allergic Reactions andAnaphylaxis . . . . . . . . . . . . 49 Gestational Anemia. 62 Transfusion-Associated Graft-vers:s-Host Disease .. .49 Sickle Cell Disease. 63 Transfusionin Special Circumstances . . . . . . . . . . . . . . 50 Thrombocytopenia in Pregnanry 63 TherapeuticApheresis ......50 Gestational Thromborytopenia . 63 Immune Thrombocytopenic Purpura. . . . . 63 Thrombotic Disorders MicroangiopathyofPregnancy . . . . . . . . . . 64 PathophysiologlofThrombosis ..... ....... 51 Thrombophilia and Venous Thromboembolism Thrombophilia .. .. ........ 51 in Pregnancy 64 ThrombophiliaTesting ......... 51 Epidemiologr, Pathophysiologr, and InheritedThrombophilias .... ......... 51 Risk Factors 64 AcquiredThrombophilias ......53 Prevention 64 Deep Venous Thrombosis and Pulmonary Embolism. . . 55 Diagnosis 65 Prevention . . ...55 Treatment. 65

AcquiredThrombophilias ......53 Prevention 64 Deep Venous Thrombosis and Pulmonary Embolism. . . 55 Diagnosis 65 Prevention . . ...55 Treatment. 65 Diagnosis ......55 Treatment. .....57 Bibliography 65 Long-termComplications..... ........58 OtherSitesofThrombosis..... .....58 Self-Assessment lest. 69 Superficial VeinThrombosis and Thrombophlebitis. . . 58 UnexplainedArterialThrombosis. ......59 Upper Efiremity Deep Venous Thrombosis. . . . . . 59 lndex 131 x